Klippel-Trénaunay syndrome: 2 Case reports and a review of genitourinary manifestations

P. D. Furness, A. Z. Barqawi, G. Bisignani, R. M. Decter

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Abstract

Purpose: We summarize the literature and present our experience with genitourinary manifestations of the Klippel-Trénaunay syndrome, which can lead to challenging management problems. Materials and Methods: We report on 2 patients with genitourinary manifestations of the Klippel-Trénaunay syndrome and performed a MEDLINE review of the literature using the key words "Klippel-Trénaunay," "vascular malformation" and "genitourinary." Results: Genitourinary manifestations were cited in 18 articles, including 1,174 cases of the Klippel-Trénaunay syndrome, detailing the presentation and management of bladder, external genitalia and retroperitoneal involvement in the Klippel-Trénaunay syndrome. The overall genitourinary symptoms in patients with the Klippel-Trénaunay syndrome seem to occur in the more severe cases and usually involve cutaneous vascular malformations of the trunk, pelvis and genitalia. Conclusions: Intra-abdominal and intrapelvic extension of the vascular malformations of the Klippel-Trénaunay syndrome frequently occurs concurrently with the lower abdominal, pelvic cutaneous involvement of the external genitalia, as in our 2 cases and in our review of the literature. These data provide a better understanding of the spectrum of genitourinary manifestations in the Klippel-Trénaunay syndrome and provide insight for the clinician to formulate individual therapies for these patients.

Original languageEnglish (US)
Pages (from-to)1418-1420
Number of pages3
JournalJournal of Urology
Volume166
Issue number4
DOIs
Publication statusPublished - Jan 1 2001

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All Science Journal Classification (ASJC) codes

  • Urology

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